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Surgical treatment of marked mitral valvar deformity combined with I-cell disease ‘Mucolipidosis II’

Published online by Cambridge University Press:  16 September 2005

Masahiro Daimon  and
Masaaki Yamagishi
Masahiro Daimon
Affiliation:
Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
Masaaki Yamagishi
Affiliation:
Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
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Abstract

I-cell disease is a rare, hereditary, and fatal disease, which is often complicated with disease of the cardiac valves. We have experienced a case of severe mitral valvar insufficiency in a 9-month-old male infant with I-cell disease. Mitral valvoplasty was difficult, because the leaflets were remarkably hypertrophic, and the tendinous cords were elongated and adherent. Despite these difficulties, surgery proved effective in the treatment of the congestive heart failure, allowing for further treatment of the I-cell disease.

Keywords

Congenital heart diseasemitral valve insufficiencymitral valve surgery
Type
Brief Report
Information
Cardiology in the Young , Volume 15 , Issue 5 , October 2005 , pp. 517 - 519
Copyright
© 2005 Cambridge University Press

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